Key result
A new syndrome characterized by secundum atrial septal defect, progressive conduction disease, left axis deviation, and unexpected death was identified in 3 of 10 families as an autosomal dominant trait.
Population
10 families in which the propositi had a secundum atrial septal defect associated with abnormal…
Design
Case_series
Authors
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Alerts clinicians to consider familial screening for conduction disease with secundum ASD; leaves open confirmation of this novel autosomal dominant syndrome.
Observational (n=61)
Identifies a novel autosomal dominant syndrome characterized by secundum atrial septal defect, progressive atrioventricular conduction disease, and risk of sudden death.
Emanuel et al. (1975) conducted an observational in Secundum atrial septal defect with abnormal atrioventricular conduction or left axis deviation (n=61). Genetic inheritance of secundum atrial septal defect with conduction abnormalities was evaluated on Presence of a new syndrome (secundum ASD, conduction disease, left axis deviation, unexpected death). A new syndrome characterized by secundum atrial septal defect, progressive conduction disease, left axis deviation, and unexpected death was identified in 3 of 10 families as an autosomal dominant trait.
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